An 11-year-old girl with Tolosa-Hunt syndrome who responded to methylprednisolone pulse therapy

Abstract

  Tolosa-Hunt syndrome (THS) is a disease characterized by posterior orbital pain with ophthalmoplegia due to nonspecific granulomatous inflammatory lesions of the cavernous sinus. The inflammation tends to improve in response to normal doses of steroid treatment. The patient was an 11-year-old girl with symptoms of ipsilateral ophthalmoplegia that appeared following posterior orbital pain on the left side. The International Headache Classification 3rd Edition beta (ICHD-3β) Tolosa-Hunt Syndrome Diagnostic Criteria (2013) was met, and a definitive diagnosis of THS was made. The usual dose of steroid treatment was ineffective, and the duration of pain-related illness was prolonged. Immediately after the start of methylprednisolone pulse therapy, the posterior orbital pain disappeared, and the symptoms of ophthalmoplegia gradually improved. There has been no recurrence during 3 years of follow-up. It was suggested that in THS cases wherein the usual dose of steroid is ineffective, methylprednisolone pulse therapy should be attempted, after excluding other diseases.